RETINAL VASCULAR ABNORMALITIES RELATED TO NEUROFIBROMATOSIS TYPE 1: Natural History and Classification by Optical Coherence Tomography Angiography in 473 Patients.
Adolescent
Choroid
/ diagnostic imaging
Female
Fluorescein Angiography
/ methods
Follow-Up Studies
Fundus Oculi
Humans
Male
Neurofibromatosis 1
/ complications
Prospective Studies
Retinal Vessels
/ abnormalities
Time Factors
Tomography, Optical Coherence
/ methods
Vascular Malformations
/ diagnosis
Visual Acuity
Journal
Retina (Philadelphia, Pa.)
ISSN: 1539-2864
Titre abrégé: Retina
Pays: United States
ID NLM: 8309919
Informations de publication
Date de publication:
01 May 2021
01 May 2021
Historique:
pubmed:
25
8
2020
medline:
15
12
2021
entrez:
25
8
2020
Statut:
ppublish
Résumé
To analyze and classify neurofibromatosis Type 1 (NF1)-related retinal vascular abnormalities (RVAs), their natural history and correlation with disease severity, in a large cohort of patients. This was an observational longitudinal study with prospective enrollment. Four hundred and seventy-three patients affected by NF1 and 150 age-matched healthy subjects were consecutively enrolled. Retinal vascular abnormalities were detected by means of near-infrared reflectance and studied by optical coherence tomography angiography. The superficial vascular plexus and the deep vascular complex (DVC) were quantitatively and qualitatively analyzed. We identified RVAs in 82 of 473 (17%) NF1 patients, but in none of the 150 healthy subjects. A comparison revealed that NF1 patients with RVAs showed a higher number of NF1 diagnostic criteria (4.3 ± 1.5 vs. 3.9 ±1.5, respectively; P = 0.02) than patients without RVAs. Three different RVA types were identified on optical coherence tomography angiography: macrovascular angiomatosis of the sole superficial vascular plexus; macrovascular angiomatosis of the superficial vascular plexus combined with microvascular angiomatosis of the deep vascular complex; and combined macrovascular angiomatosis of both superficial vascular plexus and deep vascular complex. The prospective analysis of optical coherence tomography angiography images showed no significant longitudinal evolution of RVAs (mean follow-up: 3.7 ± 2.8 years). A single patient developed a de novo single RVA, and two RVAs showed detectable changes during follow-up. In NF1 patients, RVAs are a characteristic sign that correlates with a more severe systemic disease expression, usually remaining stable during time. Optical coherence tomography angiography allows for the identification of different RVAs subtypes.
Identifiants
pubmed: 32833785
pii: 00006982-202105000-00011
doi: 10.1097/IAE.0000000000002962
doi:
Types de publication
Comparative Study
Journal Article
Observational Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
979-986Références
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